[Xanthogranuloma of the lung: a tumor-like inflammatory lesion (author's transl)].
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Biomedical subjects
Publications and source records attributed to P Godeau.
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The renin-angiotensin system was studied in 14 normotensive and hypertensive patients with scleroderma and polyarteritis nodosa (PAN). The variations in blood pressure, plasma renin activity (PRA) and serum aldosterone were compared after the Captopril test. Mean arterial pressure fell 15,7 +/- 2,6 p. 100 (p less than 0,01) in hypertensive patients, but no change was observed in normotensive subjects. PRA and serum aldosterone did not change significantly in hypertensives with scleroderma. Large variations in blood pressure, PRA and serum aldosterone were observed in 2 out of 3 patients with PAN, the third one presenting a fall in blood pressure without significant changes in PRA or serum aldosterone. These results suggest that other mechanisms are responsible for hypertension in these patients and that the Captopril acted on other factors than the renin-angiotensin system.
The authors report three cases of complete urinary retention of sudden onset during a herpes genitalis infection secondary to traumatic sexual relations. In practice, the diagnosis was made in the absence of any urological cause, the presence of local signs indicative of herpetic infection of the genital organs and lumbar puncture. Mictional disturbances disappeared in 2 to 3 weeks without leaving any sequelae. The possibility of nervous system complications in genital herpes infections has been know since 1904. By contrast, disturbances in micturition have been reported much more recently, in 1953. Complete urinary retention of sudden onset is not rare: 17 cases out of a series of 486 cases of genital infection. This may be the only clinical sign of nervous system involvement. Urinary retention develops between a few days and 3 weeks after the onset of genital herpes. Neurological examination of the lower limbs is normal. There may be transient impotence in the male and loss of the bulbo-cavernosus reflexes and impaired sensation in an S2-S3 distribution. Bladder tone is spontaneously hypoactive and increases following the injection of Urecholine. The herpes virus (HSV) is found only during the acute phase of the genital infection but almost never a few days later, when urinary retention develops.
Two cases of bullous dermatosis with dysglobulinemia are described. The first one was associated with renal and neuromuscular amyloidosis and production of a monoclonal lambda IgG. Optical and electron microscopy showed amyloid deposits beneath basal lamina of the dermis. Results for direct and indirect immunofluorescence (IF) were negative. This bullous dermatosis is not an epidermolysis bullosa acquisita (EBA), in the strict sense, because the amorphous material deposited is amyloid. In the second case, associated with Waldenström's disease, there was no cutaneous or systemic amyloidosis. Direct IF was positive; linear IgM deposits were seen along the basal membrane of the bulla and the healthy skin. Indirect IF showed the presence of circulating antibodies against basal membrane zone. This bullous dermatosis is probably an EBA, despite the absence of IgG deposits. The absence of electron microscopy does not permit the confirmation of this diagnosis.
A prospective study in 30 patients with systemic sclerosis included phonocardiographic (PCG) recordings in all cases and an echocardiogram (ECHO) in 18 patients. A specific diagnosis of myocardial fibrosis was established in 2 cases, based on anatomical criteria, and was suspected in 5 patients from combined clinical, electrical, and radiological findings. Ventricular diameters, the slope of mitral valve closure, and systolic parameters of ventricular function (haemodynamic quotient, left ventricle ejection fraction, and mean rate of circumferential shortening) were rarely abnormal, and did not correlate with possible myocardial fibrosis. Diastolic pick-up (PCG) was abnormal in 30 p. cent of the cases but this is not specific to myocardial fibrosis. Total myocardial relaxation interval, as measured by PCG, was significantly increased to 146 +/- 23 ms (mean +/- one standard deviation) in relation to a control group (p less than 0.001). A relaxation interval (measured by PCG and/or ECHO) superior to 150 ms is suggestive of myocardial fibrosis. The rate of thinning of the left ventricular posterior wall was diminished in the 14 cases in which it was measured. These results are in favour of a myocardial relaxation disorder during scleroderma.
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Dominant symptoms in 24 patients with neurological manifestations of Behçet's disease were meningo-encephalitis (13 cases), febrile meningeal syndrome (3 cases), intracranial hypertension (6 cases), polyneuritis of the lower limbs (1 case), and isolated retrobulbar ocular neuritis (1 case). Several classical notions concerning the "Neuro-Behçet" meningo-encephalitis were confirmed: the frequency of central motor lesions (33 p. cent), cerebrospinal fluid alterations (anomalies in 100 p. 100 of cases in the initial stage), and the often unfavorable course (40 p. cent). An unusual finding in this series was the frequency of cerebral veins thrombosis (4 certain and 2 probable). These could be differentiated from meningo-encephalitis by their symptomatology, predominantly that of intracranial hypertension, and by their course: often the first disorder to appear in the course of Behçet's disease, they followed a favorable neurological course but were constantly complicated by other vascular manifestations, usually phlebitis. It is suggested that a long-term anticoagulant treatment should be associated with corticoid therapy in these forms.
Raynaud's phenomenon is one of the vascular manifestations of virus B infection. It may precede or develop during cytolytic hepatitis and regresses with it. Digital necrosis may result in severe cases. The search for virus B markers should be included in the biological investigations of recent and severe Raynaud's phenomenon.
The authors report their experience of Horton's disease in 47 histologically confirmed cases treated between 1966 and 1979. The symptomatology is recalled with the incidence of the various clinical and biochemical signs. The actuarial survival curve shows a 71.2 p. cent three year and a 61 p. cent five year survival rate. There was no statistically significant difference at three years with a control population. The duration of maintenance therapy was, on average, of 24.8 months, never less than 15 months and sometimes reaching 60 months. The average maintenance dose was 12 mg. Specific and non-specific complications are discussed. The incidence of refractory and cortico-dependent forms shows that the optimal treatment for this disease has yet to be found; the often brilliant initial results of corticotherapy do not reflect its long-term efficacy.
The term of "lethal midline granuloma" has been used to apply to a number of diseases. In this paper, it is argued that this term should be only a clinical description of a relentlessly progressive destructive lesion of the nose and face. A list of all the possible causes is presented. There is a group of patients who do not correspond to any specific etiology. Reviewing the observations in the literature and our own, we think that such cases are best classified according to their histological appearance. Those without atypical cells (20% of cases) are always localized to the upper respiratory tract, while those with atypical cells (histiocytes?), 80% of cases, may become generalized and/or may evolve to a malignant lymphoma. When localized, both forms (with and without atypical cells) respond very well to high-dose radiation therapy (40 to 60 Gy). In disseminated forms or relapses following radiotherapy treatment, chemotherapy, the only available alternative treatment, has met with little success. We present the clinical and pathological features of six such patients, of whom one had no atypical cells. Radiotherapy produced a clinical remission in five of five cases, but of shorter duration than that reported in the literature. No treatment other than radiotherapy has yet proven to be regularly efficient in this rare and mutilating disease.
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One hundred and three cases of systemic lupus erythematosus (SLE) were reviewed after a period of up to 10 years. The pericardium was involved in 27%, the endocardium in 23% and the myocardium in 14.5% of the cases. Arrhythmia was rare and usually accompanied pericardial or myocardial lesions. Conduction disorders were present in 14.5% of the patients. Atrioventricular block often occurred long after the acute phase of SLE, and synthetic antimalarial drugs appear to be responsible for their development. Whether or not SLE affects the heart, the prognosis is the same during the first ten years of the disease.